Cogan's syndrome is a rare systemic disease which occurs predominantly in children and young adults. It was originally described as the combination of interstitial keratitis and audiovestibular disturbance. The nonspecific symptoms of the patients can be associated with numerous of systemic manifestations and, most characteristic, cardiovascular involvement. It affects large vessels (Takayasu-like) and medium size (polyarteritis nodosa-like) vessels. Here a case of extensive thoracoabdominal aortic replacement in a 28-year-old woman with Cogan's syndrome due to the symptomatic aortic aneurysm is described.